Note to users. If you're seeing this message, it means that your browser cannot find this page's style/presentation instructions -- or possibly that you are using a browser that does not support current Web standards. Find out more about why this message is appearing, and what you can do to make your experience of our site the best it can be.

Site Tools

  • AAAS
  • Subscribe
  • Feedback

Site Search

Search Advanced

Science 7 October 1994:
Vol. 266. no. 5182, pp. 107 - 109
DOI: 10.1126/science.7524148
Prev | Table of Contents | Next

Articles

Science, Vol 266, Issue 5182, 107-109
Copyright © 1994 by American Association for the Advancement of Science

articles

Cystic fibrosis heterozygote resistance to cholera toxin in the cystic fibrosis mouse model

SE Gabriel, KN Brigman, BH Koller, RC Boucher, and MJ Stutts

Department of Medicine, University of North Carolina, Chapel Hill 27599.

The effect of the number of cystic fibrosis (CF) alleles on cholera toxin (CT)-induced intestinal secretion was examined in the CF mouse model. CF mice that expressed no CF transmembrane conductance regulator (CFTR) protein did not secrete fluid in response to CT. Heterozygotes expressed 50 percent of the normal amount of CFTR protein in the intestinal epithelium and secreted 50 percent of the normal fluid and chloride ion in intestinal epithelium and secreted 50 percent of the normal fluid and chloride ion and fluid secretion suggests that CF heterozygotes might possess a selective advantage of resistance to cholera.


THIS ARTICLE HAS BEEN CITED BY OTHER ARTICLES:
Gating of the CFTR Cl\#8722; channel by ATP-driven nucleotide-binding domain dimerisation.
T.-C. Hwang and D. N. Sheppard (2009)
J. Physiol. 587, 2151-2161
   Abstract »    Full Text »    PDF »
Detection of Epidermal Thickening in GJB2 Carriers with Epidermal US.
P. Guastalla, V. I. Guerci, A. Fabretto, F. Faletra, D. L. Grasso, E. Zocconi, D. Stefanidou, P. D'Adamo, L. Ronfani, M. Montico, et al. (2009)
Radiology 251, 280-286
   Abstract »    Full Text »    PDF »
Hypoxia inducible factor-1 (HIF-1)-mediated repression of cystic fibrosis transmembrane conductance regulator (CFTR) in the intestinal epithelium.
W. Zheng, J. Kuhlicke, K. Jackel, H. K. Eltzschig, A. Singh, M. Sjoblom, B. Riederer, C. Weinhold, U. Seidler, S. P. Colgan, et al. (2009)
FASEB J 23, 204-213
   Abstract »    Full Text »    PDF »
Deletion of the chloride transporter Slc26a9 causes loss of tubulovesicles in parietal cells and impairs acid secretion in the stomach.
J. Xu, P. Song, M. L. Miller, F. Borgese, S. Barone, B. Riederer, Z. Wang, S. L. Alper, J. G. Forte, G. E. Shull, et al. (2008)
PNAS 105, 17955-17960
   Abstract »    Full Text »    PDF »
Three-dimensional Reconstruction of Human Cystic Fibrosis Transmembrane Conductance Regulator Chloride Channel Revealed an Ellipsoidal Structure with Orifices beneath the Putative Transmembrane Domain.
K. Mio, T. Ogura, M. Mio, H. Shimizu, T.-C. Hwang, C. Sato, and Y. Sohma (2008)
J. Biol. Chem. 283, 30300-30310
   Abstract »    Full Text »    PDF »
Functional and Structural Implications of the Complement Factor H Y402H Polymorphism Associated with Age-Related Macular Degeneration.
R. J. Ormsby, S. Ranganathan, J. C. Tong, K. M. Griggs, D. P. Dimasi, A. W. Hewitt, K. P. Burdon, J. E. Craig, J. Hoh, and D. L. Gordon (2008)
Invest. Ophthalmol. Vis. Sci. 49, 1763-1770
   Abstract »    Full Text »    PDF »
Fluid Secretion Caused by Aerolysin-Like Hemolysin of Aeromonas sobria in the Intestines Is Due to Stimulation of Production of Prostaglandin E2 via Cyclooxygenase 2 by Intestinal Cells.
Y. Fujii, K. Tsurumi, M. Sato, E. Takahashi, and K. Okamoto (2008)
Infect. Immun. 76, 1076-1082
   Abstract »    Full Text »    PDF »
Cystic Fibrosis: Lessons from the Sweat Gland.
P. M. Quinton (2007)
Physiology 22, 212-225
   Abstract »    Full Text »    PDF »
Evaluating candidate agents of selective pressure for cystic fibrosis.
E. M Poolman and A. P Galvani (2007)
J R Soc Interface 4, 91-98
   Abstract »    Full Text »    PDF »
Changes in disease gene frequency over time with differential genotypic fitness and various control strategies.
P. N. Thompson, J. A. P. Heesterbeek, and J. A. M. van Arendonk (2006)
J Anim Sci 84, 2629-2635
   Abstract »    Full Text »    PDF »
Inaugural Article: Calcium-sensing receptor abrogates secretagogue- induced increases in intestinal net fluid secretion by enhancing cyclic nucleotide destruction.
J. Geibel, K. Sritharan, R. Geibel, P. Geibel, J. S. Persing, A. Seeger, T. K. Roepke, M. Deichstetter, C. Prinz, S. X. Cheng, et al. (2006)
PNAS 103, 9390-9397
   Abstract »    Full Text »    PDF »
Mutual Enhancement of Virulence by Enterotoxigenic and Enteropathogenic Escherichia coli.
J. K. Crane, S. S. Choudhari, T. M. Naeher, and M. E. Duffey (2006)
Infect. Immun. 74, 1505-1515
   Abstract »    Full Text »    PDF »
Differential Sensitivity of the Cystic Fibrosis (CF)-associated Mutants G551D and G1349D to Potentiators of the Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) Cl- Channel.
Z. Cai, A. Taddei, and D. N. Sheppard (2006)
J. Biol. Chem. 281, 1970-1977
   Abstract »    Full Text »    PDF »
Estrogen-Induced Abnormally High Cystic Fibrosis Transmembrane Conductance Regulator Expression Results in Ovarian Hyperstimulation Syndrome.
L. C. Ajonuma, L. L. Tsang, G. H. Zhang, C. H. Y. Wong, M. C. Lau, L. S. Ho, D. K. Rowlands, C. X. Zhou, C. P. Ng, J. Chen, et al. (2005)
Mol. Endocrinol. 19, 3038-3044
   Abstract »    Full Text »    PDF »
Lysophosphatidic acid inhibits cholera toxin-induced secretory diarrhea through CFTR-dependent protein interactions.
C. Li, K. S. Dandridge, A. Di, K. L. Marrs, E. L. Harris, K. Roy, J. S. Jackson, N. V. Makarova, Y. Fujiwara, P. L. Farrar, et al. (2005)
J. Exp. Med. 202, 975-986
   Abstract »    Full Text »    PDF »
Reversible Silencing of CFTR Chloride Channels by Glutathionylation.
W. Wang, C. Oliva, G. Li, A. Holmgren, C. H. Lillig, and K. L. Kirk (2005)
J. Gen. Physiol. 125, 127-141
   Abstract »    Full Text »    PDF »
Interleukin 8 Secretion from Monocytes of Subjects Heterozygous for the {Delta}F508 Cystic Fibrosis Transmembrane Conductance Regulator Gene Mutation Is Altered.
M. M. Zaman, A. Gelrud, O. Junaidi, M. M. Regan, M. Warny, J. C. Shea, C. Kelly, B. P. O'Sullivan, and S. D. Freedman (2004)
Clin. Vaccine Immunol. 11, 819-824
   Abstract »    Full Text »    PDF »
Capsaicin Potentiates Wild-Type and Mutant Cystic Fibrosis Transmembrane Conductance Regulator Chloride-Channel Currents.
T. Ai, S. G. Bompadre, X. Wang, S. Hu, M. Li, and T.-C. Hwang (2004)
Mol. Pharmacol. 65, 1415-1426
   Abstract »    Full Text »    PDF »
Genetic dissection of myocilin glaucoma.
G. Gong, O. Kosoko-Lasaki, G. R. Haynatzki, and M. R. Wilson (2004)
Hum. Mol. Genet. 13, R91-102
   Abstract »    Full Text »    PDF »
Inhibitory Regulation of Cystic Fibrosis Transmembrane Conductance Regulator Anion-transporting Activities by Shank2.
J. Y. Kim, W. Han, W. Namkung, J. H. Lee, K. H. Kim, H. Shin, E. Kim, and M. G. Lee (2004)
J. Biol. Chem. 279, 10389-10396
   Abstract »    Full Text »    PDF »
Identification and Molecular Characterization of EatA, an Autotransporter Protein of Enterotoxigenic Escherichia coli.
S. K. Patel, J. Dotson, K. P. Allen, and J. M. Fleckenstein (2004)
Infect. Immun. 72, 1786-1794
   Abstract »    Full Text »    PDF »
Association of Cystic Fibrosis with Abnormalities in Fatty Acid Metabolism.
S. D. Freedman, P. G. Blanco, M. M. Zaman, J. C. Shea, M. Ollero, I. K. Hopper, D. A. Weed, A. Gelrud, M. M. Regan, M. Laposata, et al. (2004)
N. Engl. J. Med. 350, 560-569
   Abstract »    Full Text »    PDF »
Pathophysiology and Management of Pulmonary Infections in Cystic Fibrosis.
R. L. Gibson, J. L. Burns, and B. W. Ramsey (2003)
Am. J. Respir. Crit. Care Med. 168, 918-951
   Abstract »    Full Text »    PDF »
A Scientific Koan: What Is the Cause of Cystic Fibrosis?.
B. K. Rubin (2003)
Chest 123, 1792-1793
   Full Text »    PDF »
Studies of the Mechanism of Action of the Aerolysin-Like Hemolysin of Aeromonas sobria in Stimulating T84 Cells To Produce Cyclic AMP.
Y. Fujii, T. Nomura, R. Yokoyama, S. Shinoda, and K. Okamoto (2003)
Infect. Immun. 71, 1557-1560
   Abstract »    Full Text »    PDF »
cAMP-dependent exocytosis and vesicle traffic regulate CFTR and fluid transport in rat jejunum in vivo.
N. A. Ameen, C. Marino, and P. J. I. Salas (2003)
Am J Physiol Cell Physiol 284, C429-C438
   Abstract »    Full Text »    PDF »
Indazole Inhibition of Cystic Fibrosis Transmembrane Conductance Regulator Cl- Channels in Rat Epididymal Epithelial Cells.
X.D. Gong, P. Linsdell, K.H. Cheung, G.P.H. Leung, and P.Y.D. Wong (2002)
Biol Reprod 67, 1888-1896
   Abstract »    Full Text »    PDF »
The allelic architecture of human disease genes: common disease-common variant... or not?.
J. K. Pritchard and N. J. Cox (2002)
Hum. Mol. Genet. 11, 2417-2423
   Abstract »    Full Text »    PDF »
CFTR chloride channels are regulated by a SNAP-23/syntaxin 1A complex.
E. Cormet-Boyaka, A. Di, S. Y. Chang, A. P. Naren, A. Tousson, D. J. Nelson, and K. L. Kirk (2002)
PNAS 99, 12477-12482
   Abstract »    Full Text »    PDF »
Upregulation of CFTR expression but not SLC26A3 and SLC9A3 in ulcerative colitis.
H. Lohi, S. Makela, K. Pulkkinen, P. Hoglund, M.-L. Karjalainen-Lindsberg, P. Puolakkainen, and J. Kere (2002)
Am J Physiol Gastrointest Liver Physiol 283, G567-G575
   Abstract »    Full Text »    PDF »
Phloxine B Interacts with the Cystic Fibrosis Transmembrane Conductance Regulator at Multiple Sites to Modulate Channel Activity.
Z. Cai and D. N. Sheppard (2002)
J. Biol. Chem. 277, 19546-19553
   Abstract »    Full Text »    PDF »
Lung Infections Associated with Cystic Fibrosis.
J. B. Lyczak, C. L. Cannon, and G. B. Pier (2002)
Clin. Microbiol. Rev. 15, 194-222
   Abstract »    Full Text »    PDF »
Electrolyte Transport in the Mammalian Colon: Mechanisms and Implications for Disease.
K. Kunzelmann and M. Mall (2002)
Physiol Rev 82, 245-289
   Abstract »    Full Text »    PDF »
A cluster of negative charges at the amino terminal tail of CFTR regulates ATP-dependent channel gating.
J. Fu, H.-L. Ji, A. P Naren, and K. L Kirk (2001)
J. Physiol. 536, 459-470
   Abstract »    Full Text »    PDF »
Interleukin-5 inhibition of biliary cell chloride currents and bile flow.
J. M. McGill, M. S. Yen, O. W. Cummings, G. Alpini, G. LeSage, K. E. Pollok, B. Miller, S. K. Engle, and A. P. Stansfield (2001)
Am J Physiol Gastrointest Liver Physiol 280, G738-G745
   Abstract »    Full Text »    PDF »
Inhibition of enterotoxin-induced porcine colonic secretion by diarylsulfonylureas in vitro.
E. K. O'Donnell, R. L. Sedlacek, A. K. Singh, and B. D. Schultz (2000)
Am J Physiol Gastrointest Liver Physiol 279, G1104-G1112
   Abstract »    Full Text »    PDF »
Forskolin-induced apical membrane insertion of virally expressed, epitope-tagged CFTR in polarized MDCK cells.
M. Howard, X. Jiang, D. B. Stolz, W. G. Hill, J. A. Johnson, S. C. Watkins, R. A. Frizzell, C. M. Bruton, P. D. Robbins, and O. A. Weisz (2000)
Am J Physiol Cell Physiol 279, C375-C382
   Abstract »    Full Text »    PDF »
Two mechanisms of genistein inhibition of cystic fibrosis transmembrane conductance regulator Cl- channels expressed in murine cell line.
K A Lansdell, Z Cai, J F Kidd, and D N Sheppard (2000)
J. Physiol. 524, 317-330
   Abstract »    Full Text »    PDF »
Cystic Fibrosis Transmembrane Conductance Regulator. STRUCTURE AND FUNCTION OF AN EPITHELIAL CHLORIDE CHANNEL.
M. H. Akabas (2000)
J. Biol. Chem. 275, 3729-3732
   Full Text »    PDF »
Permeation through the CFTR chloride channel.
N. McCarty (2000)
J. Exp. Biol. 203, 1947-1962
   Abstract »    PDF »
CFTR Chloride Channel Regulation by an Interdomain Interaction.
A. P. Naren, E. Cormet-Boyaka, J. Fu, M. Villain, J. E. Blalock, M. W. Quick, and K. L. Kirk (1999)
Science 286, 544-548
   Abstract »    Full Text »
A novel plant-derived inhibitor of cAMP-mediated fluid and chloride secretion.
S. E. Gabriel, S. E. Davenport, R. J. Steagall, V. Vimal, T. Carlson, and E. J. Rozhon (1999)
Am J Physiol Gastrointest Liver Physiol 276, G58-G63
   Abstract »    Full Text »    PDF »
Physiological Basis of Cystic Fibrosis: A Historical Perspective.
P. M. QUINTON (1999)
Physiol Rev 79, 3-22
   Abstract »    Full Text »    PDF »
Control of CFTR Channel Gating by Phosphorylation and Nucleotide Hydrolysis.
D. C. GADSBY and A. C. NAIRN (1999)
Physiol Rev 79, 77-107
   Abstract »    Full Text »    PDF »
Pathophysiology of Gene-Targeted Mouse Models for Cystic Fibrosis.
B. R. GRUBB and R. C. BOUCHER (1999)
Physiol Rev 79, 193-214
   Abstract »    Full Text »    PDF »
Pharmacogenetics in Biological Perspective.
W. Kalow (1997)
Pharmacol. Rev. 49, 369-380
   Abstract »    Full Text »    PDF »
Evidence for a Heterozygote Advantage in Congenital Adrenal Hyperplasia due to 21-Hydroxylase Deficiency.
S. F. Witchel, P. A. Lee, M. Suda-Hartman, M. Trucco, and E. P. Hoffman (1997)
J. Clin. Endocrinol. Metab. 82, 2097-2101
   Abstract »    Full Text »    PDF »
Delta F508 in cystic fibrosis: willing but not able.
K. W Southern (1997)
Arch. Dis. Child. 76, 278-282
   Full Text »
Enamel Mineral Composition of Normal and Cystic Fibrosis Transgenic Mice.
J.T. Wright, K.I. Hall, and B.R. Grubb (1996)
Advances in Dental Research 10, 270-275
   Abstract »    PDF »
Infection Emergent.
J. Lederberg (1996)
JAMA 275, 243-245
   Abstract »    PDF »
Regulation of ion channels by ABC transporters that secrete ATP.
Q al-Awqati (1995)
Science 269, 805-806
   PDF »
Gastrointestinal Cancer and the Cystic Fibrosis Gene.
M. W. Audeh, J. P. Neglia, S. C. FitzSimmons, A. B. Lowenfels, and The Cystic Fibrosis and Cancer Study Group (1995)
N. Engl. J. Med. 333, 129-130
   Full Text »
Cysteine Substitutions Reveal Dual Functions of the Amino-terminal Tail in Cystic Fibrosis Transmembrane Conductance Regulator Channel Gating.
J. Fu and K. L. Kirk (2001)
J. Biol. Chem. 276, 35660-35668
   Abstract »    Full Text »    PDF »


To Advertise     Find Products

Science. ISSN 0036-8075 (print), 1095-9203 (online)